Membranous Obstruction of the Inferior Vena Cava

Abstract

Membranous obstruction of the inferior vena cava (MOVC) is a rare clinical entity. The incidence is higher in Japan and Africa than that in USA and Europe. MOVC is considered to be different from the Budd-Chiari syndrome. There are differences in clinical manifestation and pathologic changes in the liver. Findings of the liver revealed cirrhosis in all patients but in one. Ascites and esophageal varices could be observed in 79% and in 58% of the patients, respectively. According to Sugiura's classification, they consisted of Type Ia in 63%, Type Ib in 11%, Type II in 11%, and Type III in 16%. Portopulmonary shunt by splenopneumopexy was successfully performed on 19 patients with MOVC in this clinic, of which diagnosis was established by cavography.

Membranous Budd–Chiari syndrome in Caucasians

“…Patient characteristics at initial presentation. (Bracco International B.V., Amsterdam, The Netherlands), a second-generation ultrasound contrast agent, was used [5]. Hepatovenography was performed as the gold standard to diagnose membranous BCS.…”

“…Congenital membranous BCS can be classified into three different types [5]. While Type I is characterized by a thin membrane at the vena cava or atrium, Type II is distinguished by an absent segment of the vena cava with an occluded hepatic vein.…”

Sinusoidal Hypertension: A Cause of Liver Cirrhosis in Developing Countries

“…It was reported from many countries of Asia and Africa [11,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29]. High incidence of cirrhosis and hepatocellular carcinoma (HCC) was reported in the disease from Japan [15][16][17][18][19] and South Africa [27,28]. HVCS was previously considered a congenital vascular malformation, and diagnosed late in persons with complete obstruction of IVC by cavogram or liver biopsy or detected at autopsy.…”

“…The incidence of cirrhosis HVCS in reported series varied from 27 to 100% [12,13,[15][16][17][18][19]25,26]. Kage, et al [57] who studied livers and inferior vena cava from 17 autopsied cases of HVCS observed that hepatic veins of different sizes and portal veins were involved in all, and the occluding lesion or stenosis both in the IVC and intrahepatic veins were due to thrombosis and its squeal.…”

“…HVCS was frequently reported from Japan in early 20 th century [15][16][17][18][19] and from Europe and North America in 19 th and 20 th century [69][70][71][72][73][74]. This disease has now almost disappeared from these areas.…”